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A knock‐in mouse line conditionally expressing the tumor suppressor WTX/AMER1
Authors:Agnès Boutet  Glenda Comai  Aurélie Charlet  Fariba Jian Motamedi  Haroun Dhib  Roberto Bandiera  Andreas Schedl
Institution:Université C?te d'Azur, Inserm U1091, CNRS UMR 7277, iBV, France
Abstract:WTX/AMER1 is an important developmental regulator, mutations in which have been identified in a proportion of patients suffering from the renal neoplasm Wilms' tumor and in the bone malformation syndrome Osteopathia Striata with Cranial Sclerosis (OSCS). Its cellular functions appear complex and the protein can be found at the membrane, within the cytoplasm and the nucleus. To understand its developmental and cellular function an allelic series for Wtx in the mouse is crucial. Whereas mice carrying a conditional knock out allele for Wtx have been previously reported, a gain‐of‐function mouse model that would allow studying the molecular, cellular and developmental role of Wtx is still missing. Here we describe the generation of a novel mouse strain that permits the conditional activation of WTX expression. Wtx fused to GFP was introduced downstream a stop cassette flanked by loxP sites into the Rosa26 locus by gene targeting. Ectopic WTX expression is reported after crosses with several Cre transgenic mice in different embryonic tissues. Further, functionality of the fusion protein was demonstrated in the context of a Wtx null allele.
Keywords:embryogenesis  knock‐in  mendelian disorders  mouse  Rosa26 locus  WTX/AMER1
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